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Usefulness and limitations of 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy in the aetiological diagnosis of amyloidotic cardiomyopathy

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Abstract

Purpose

We previously reported in a small series of patients that 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) scintigraphy tested positive in transthyretin-related (TTR) (both mutant and wild-type) but not in primary (AL) amyloidotic cardiomyopathy (AC). We extended our study to a larger cohort of patients with AC.

Methods

We evaluated (1) 45 patients with TTR-related AC (28 mutant and 17 wild-type), (2) 34 with AL-related AC and (3) 15 non-affected controls. Myocardial uptake of 99mTc-DPD (740 MBq i.v.) was semiquantitatively and visually assessed at 5 min and at 3 h.

Results

Heart retention (HR) and heart to whole-body retention ratio (H/WB) of late 99mTc-DPD uptake were higher among TTR-related AC (HR 7.8%; H/WB 10.4) compared with both unaffected controls (HR 3.5%; H/WB 5.7; p < 0.0001) and AL-related AC (HR 4.0%; H/WB 6.1; p < 0.0001). For the diagnosis of TTR-related AC, positive and negative predictive accuracy of visual scoring of cardiac retention were: 80 and 100% (visual score ≥1); 88 and 100% (visual score ≥2); and 100 and 68% (visual score = 3). At adjusted linear regression analysis, TTR aetiology turned out to be the only positive predictor of increasing 99mTc-DPD uptake in terms of both HR [β 2.5, 95% confidence interval (CI) 1.5–3.5; p < 0.0001] and H/WB (β 3.5, 95% CI 2.1–4.9; p < 0.0001).

Conclusion

While 99mTc-DPD scintigraphy was confirmed to be useful for differentiating TTR from AL-related AC, diagnostic accuracy was lower than previously reported due to a mild degree of tracer uptake in about one third of AL patients. 99mTc-DPD scintigraphy can provide an accurate differential diagnosis in cases of absent or intense uptake evaluated by visual score.

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Authors and Affiliations

  1. Institute of Cardiology, University of Bologna and Policlinico S. Orsola-Malpighi Hospital, via Massarenti 9, 40138, Bologna, Italy

    Claudio Rapezzi, Candida Cristina Quarta, Simone Longhi, Pamela Gallo, Christian Gagliardi & Angelo Branzi

  2. Nuclear Medicine Unit, S. Orsola-Malpighi Hospital, Bologna, Italy

    Pier Luigi Guidalotti & Cinzia Pettinato

  3. Department of Pathology, University of Bologna and S. Orsola-Malpighi Hospital, Bologna, Italy

    Ornella Leone

  4. Department of Diagnostic and Experimental Medicine, Section of Medical Genetics, University of Ferrara, Ferrara, Italy

    Alessandra Ferlini

  5. Department of Neurology, Ospedale Bellaria, Bologna, Italy

    Fabrizio Salvi

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  1. Claudio Rapezzi
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Correspondence to Claudio Rapezzi.

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Rapezzi, C., Quarta, C.C., Guidalotti, P.L. et al. Usefulness and limitations of 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy in the aetiological diagnosis of amyloidotic cardiomyopathy. Eur J Nucl Med Mol Imaging 38, 470–478 (2011). https://doi.org/10.1007/s00259-010-1642-7

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